Report | Question ID | Question | Discussion | Answer | Year |
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20160075 | MP/H Rules/Histology--Breast: What histology code(s) and MP/H rule applies for a breast resection final diagnosis of "undifferentiated sarcoma associated with a malignant phyllodes tumor and a tumor size of approximately 7 x 6.5 x 4 cm"? (The tumor is primarily sarcoma, with the phyllodes tumor measuring 2.8 cm)? See Discussion. |
Patient has a diagnosis of undifferentiated sarcoma with an associated malignant phyllodes tumor in a single mass. Should this be abstracted as two primaries, one for an undifferentiated sarcoma and the other for a malignant phyllodes tumor? Which MP/H rule applies? |
Abstract a single primary. Based on the information provided, this is a single tumor, and therefore a single primary, Rule M3. Code the histology to malignant phyllodes tumor. According to our expert pathologist consultant, "The presence of a phyllodes tumor component identifies the whole thing as such. Stromal overgrowth/sarcoma is the usual identifier of malignancy in a phyllodes tumor. (If there were no phyllodes component we would be left with undifferentiated sarcoma, but that is not the case here. The diagnosis of malignancy in phyllodes tumor may be difficult/problematic when there is no overt stromal/sarcoma overgrowth as in this case.) As an aside, the behaviors of pure sarcoma and a phyllodes tumor such as we have here are similar, but we would lose the primary diagnosis if we just called this sarcoma." |
2016 |
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20220003 | Reportability/Histology--Anus: Are 2021 diagnoses of anal intraepithelial neoplasia (AIN) II or AIN II-III reportable in patients with a known history of AIN II or AIN II-III diagnosed prior to 2021? See Discussion. |
Patient has a history of AIN I/low-grade squamous intraepithelial lesion (LSIL) dating back to at least 2015, was diagnosed with AIN II-III in 12/2019, and then diagnosed again with AIN II-III in 08/2021. There is no indication of treatment or a disease-free interval for this patient. SINQ 20210015, while not an exact match to this case, implies there is no clear disease-free interval for these AIN diagnoses, so it is the same non-reportable neoplasm diagnosed prior to reportability (12/2019). However, there was a diagnosis of a reportable neoplasm in 2021, so it also seems possible this would be accessioned as a reportable tumor based on a diagnosis of reportable tumor diagnosis in 2021. With the reportability changes for these intraepithelial neoplasia II/II-III tumors, these situations will arise more frequently. |
Report AIN II and AIN II-III cases when initially diagnosed in 2021 or later. Do not report retrospective cases; that is, cases with diagnoses prior to 2021 with continuation of AIN II or AIN II-III extending into the reportable period. |
2022 |
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20210002 | Multiple Primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient diagnosed with therapy-related myelodysplastic syndrome (t-MDS) (9920/3) in 2015 followed by a 2020 diagnosis of myelodysplastic syndrome, NOS (MDS, NOS) (9989/3)? See Discussion. |
Patient has a history of B-cell lymphoma with multimodality treatment in 2002. Lab work in 2015 showed multilineage dysplasia leading to a diagnosis of therapy-related myelodysplastic syndrome. Patient presents in 2020 for a bone marrow biopsy now showing low-grade MDS. The MDS appears to have the same multilineage dysplasia previously identified. MDS, NOS is not listed in the Heme DB as a possible transformation of t-MDS, nor is it listed as a Same Primary for t-MDS. Likewise, t-MDS is not listed as a more specific myelodysplastic syndrome, a transformation of MDS NOS, or a Same Primary as MDS, NOS. The first M rule that applies to this case is M15, and the Multiple Primaries Calculator indicates that the MDS, NOS should be a new primary. |
Abstract separate primaries using Rule M15 of the Hematopoietic and Lymphoid Neoplasms (Heme) Manual. The Heme Database states: Excluded from this category are progression of myeloproliferative neoplasms (MPNs) and evolution of primary MDS or primary MDS/MPN to acute myeloid leukemia (AML); in each of these latter cases evolution to AML is part of the natural history of the primary disease and it may be impossible to distinguish natural progression from therapy-related changes. There is no indication of transformation. |
2021 |
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20190064 | Multiple Primaries--Heme & Lymphoid Neoplasms: Patient is diagnosed with myelodysplastic syndrome (MDS) with an early/evolving acute myeloid leukemia (AML) thought to be treatment related. Does rule M11 apply since there are two biopsies within 21 days, and therefore, two primaries, or one primary (9920/3)? See Discussion. |
Patient has a history of breast cancer and diffuse large B-cell lymphoma (DLBCL), both treated with chemotherapy and radiation. On 6/26/19, bone marrow biopsy: MDS with excess blasts-2 (18% dysplastic blasts) in a normocellular marrow (overall 40% cellularity) with trilineage dysplasia. Comment: least myelodysplastic syndrome with excess blasts-2. However, an early/evolving AML cannot be completely excluded. The findings likely represent therapy-related myeloid neoplasm. MD note on 7/15/19: Diagnosis: MDS, high grade borderline AML with complex karyotype secondary disease. Patient has high grade MDS which is bordering on AML transformation with 20% blasts by IHC and areas higher than this. This is likely secondary to the treatment she has received for her other cancers particularly pelvic radiation for her DLBCL. Given her very high IPSS score, it is likely she will eventually develop AML. No treatment given. On 7/15/19, bone marrow biopsy: Persistent acute leukemia in a marrow with trilineage dyspoiesis and 23% blasts. |
Code as one primary (9920/3). This case does not fit the rules very well, since it is a treatment-related neoplasm and involves a transformation of MDS to AML during the clinical workup. Per the abstractor notes for 9920/3, code 9920/3 when the physician comments that the neoplasm is treatment related. This can be for the MDS or the AML. Use text fields to document that it was first referred to as MDS and then transformed to AML. If you followed the rules strictly and coded this as two primaries (the MDS and AML), you would lose the information that this was treatment related, which is more important. |
2019 |
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20120039 | Primary site--Heme & Lymphoid Neoplasms: What primary site and heme rule applies when a PET scan shows bilateral renal masses, hypermetabolic liver lesions and retroperitoneal lymphadenopathy, a right kidney biopsy was positive for diffuse large B-cell lymphoma, and the bone marrow biopsy was negative? See Discussion. |
Patient has a history of chronic lymphocytic leukemia (CLL). February 2011 abdomen/pelvis x-ray showed development of bilateral renal masses. April 2011 PET scan showed intense areas of hypermetabolic activity corresponding to known bilateral renal masses, new hypermetabolic liver lesions, as well as left upper retroperitoneal lymphadenopathy. All findings are worrisome for malignancy. March 2011 right kidney mass biopsy was positive for diffuse large B-cell lymphoma (DLBCL). Bone marrow biopsy was negative for lymphoma. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. Per Rule PH25, code the primary site of the diffuse large B-cell lymphoma to C649 (kidneys) and laterality to 4 (bilateral). Per PH25, code the primary site to the organ when a lymphoma is present in an and that . This patient had involvement of an organ (bilateral kidneys) as well as regional lymph nodes for that organ. The retroperitoneal lymph nodes are regional for the kidney. The diffuse large B-cell lymphoma is an acute transformation of the chronic lymphocytic leukemia. Because the DLBCL occurred more than 21 days after the CLL, it is a new primary per Rule M10. SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx. |
2012 |
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20110126 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be accessioned, and what rule applies, when the patient has a history of diffuse large B-cell lymphoma diagnosed in 2003, a follicular lymphoma diagnosed in 2009, and another diagnosis of follicular lymphoma in 2010? Is the application of the multiple primary rules effected if it is unknown whether the patient was ever disease free? See Discussion. | Patient has a history of diffuse large B-cell lymphoma involving multiple lymph node regions (site C778) with bone marrow involvement diagnosed in 2003 and a history of follicular lymphoma confined to the thyroid and neck lymph nodes diagnosed in 2009. In 2010 the patient was diagnosed with follicular lymphoma in the inguinal and abdominal lymph nodes.
The 2003 diagnosis of DLBCL and the 2009 diagnosis of follicular lymphoma are the same primary according to the 2009 rules, the Single Versus Subsequent Primaries Table.
What rule is used to determine whether the 2010 diagnosis of follicular lymphoma represents a new primary? Which histologies are compared using the rules: the 2010 follicular lymphoma diagnosis to the 2009 follicular diagnosis or the 2010 follicular lymphoma diagnosis to the 2003 DLBCL diagnosis? |
This case should be accessioned as one primary.
Reportability is determined by the year of diagnosis. The original DLBCL was diagnosed in 2003 and the follicular lymphoma in 2009. The pre-2010 rules are used for both cases. Per the Single Versus Subsequent Primaries Table, these are the same primary. It is reported with the histology 9680/3 [diffuse large B-cell lymphoma]
Do not compare the DLBCL diagnosed in 2003 and the follicular lymphoma diagnosed in 2010 because the determination of the number of primaries for the two specific histologic types was done (as it should have been) using the rules in effect in 2009 when the follicular lymphoma was first diagnosed. The determination of a single or multiple primaries is made the first time the patient presents with the two different diseases; it is not changed when the same disease process reappears after 2010. |
2011 |
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20100083 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient with a longstanding history of follicular cell non-Hodgkin lymphoma followed by a 2010 diagnosis of "B-cell lymphoma with prominent large cell component, compatible with primary cutaneous follicle center cell lymphoma"? See Discussion. | Patient has a history of follicular cell non-Hodgkin lymphoma dating back to the 1990s. The patient was treated with chemotherapy and bone marrow transplantation, radiation and rituximab. The patient had no evidence of recurrence. In April 2010 a lesion appeared on the side of the scalp above the left ear with a diagnosis of "B-cell lymphoma with prominent large cell component, compatible with primary cutaneous follicle center cell lymphoma." The oncology diagnosis is "primary cutaneous follicle center lymphoma."
Would the Multiple Primaries Calculator be used in this case? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Accession two primaries per Rule M15. Per the Multiple Primaries Calculator, primary cutaneous follicle center lymphoma [9597/3] following a diagnosis of follicular lymphoma, NOS [9690/3] is a new primary.
While the pathologic diagnosis was B-cell lymphoma "compatible with" primary cutaneous follicle center cell lymphoma and ambiguous terms cannot be used to identify a more specific histology, the physician confirmed the more specific diagnosis without ambiguous terminology. Therefore, this diagnosis should be coded.
SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx. |
2010 |
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20230056 | Reportability/Histology--Heme and Lymphoid Neoplasms: What is the histology code for nodular lymphocyte predominant B cell lymphoma that is never called Hodgkin lymphoma? Is it acceptable to record the histology code for nodular lymphocyte predominant Hodgkin lymphoma, (9659/3)? See Discussion. |
Patient has a history of human immunodeficiency virus and diffuse large B cell lymphoma diagnosed in 2012, and is status/post systemic therapy and in remission since completing first course treatment. In 2022, the patient has imaging suspicious for recurrence. A biopsy of a deep left cervical lymph node showed atypical lymphoid infiltrate with the comment: “This is a challenging case. The constellation of findings is most in keeping with early / focal and subtle involvement by a nodular lymphocyte predominant B-cell lymphoma. We find no evidence of involvement by a diffuse large B-cell lymphoma.” The managing physician later states, “Cervical lymph node biopsy (06/2022) was consistent with nodular lymphocyte predominant B cell lymphoma.” |
According to the 5th edition WHO Blue Book for Hematopoietic Neoplasms, Beta Version, (not released yet), nodular lymphocyte predominant B-cell lymphoma is an alternate name for 9659/3. We will update the Heme database once the 5th edition is released in print. |
2023 |
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20160053 | MP/H Rules/Histology: How is the histology coded for an invasive adenocarcinoma arising in a papilloma with high-grade dysplasia? See Discussion. |
Patient has a perihilar bile duct primary with a microscopic focus of invasive moderately differentiated adenocarcinoma arising in a large papilloma. The MP/H Rules do not address adenocarcinomas arising in a papilloma, only adenocarcinomas arising in an adenoma (or polyp). Should the histology be coded as 8140 for the invasive adenocarcinoma component? Or should the matrix principle be applied and the histology coded as a malignant glandular papilloma (8260/3)? |
Assign 8503/3 for invasive adenocarcinoma arising in a papilloma with high-grade dysplasia, perihilar bile duct primary. Neither ICD-O-3 nor the WHO classification have a code for this specific histology; however, our expert pathologist consultant states 8503/3 is the best available choice based on pages 264 and 273 in the WHO Digestive system classification. |
2016 |
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20220006 | Histology/Brain and CNS: How is histology coded for a 2021 diagnosis of “neuroepithelial tumor with PATZ1-EWSR1 fusion, not elsewhere classified” found during a right thalamic mass resection? See Discussion. |
Patient has a remote history of a right thalamic mass status-post two resections; reported as malignant oligodendroglioma (pathology not received) and chemo/radiation therapy, who recently presented with persistent headaches. Imaging revealed a 3.4 cm heterogeneous lobulated right thalamic mass with coarse calcifications and a probable cystic component. Pathologist indicates the histologic and immunophenotypic features of this neoplasm are that of relatively circumscribed neuroepithelial tumor without high grade features (mitotic activity, microvascular proliferation, necrosis). Molecularly this neoplasm is characterized by a PATZ1-EWSR1 fusion, which has recently been proposed to be a distinct neuroepithelial tumor entity with a broad histological spectrum. |
Assign 8000/1. Neuroepithelial tumor with PATZ1-EWSR1 fusion, not elsewhere classified, is not recognized as a distinct entity at this time. It is not listed in ICD-O-3.2 or in the 5th edition of the WHO CNS classification. |
2022 |